Lymphomas are defined as heterogeneous malignancies of the lymphatic system characterized by a proliferation of lymphoid cells or their precursors. Malignant lymphoma of the oral cavity has been described previously although reports on the occurrence of intraoral extranodal T-cell lymphomas are scarce. Oral lesions may appear as a painless enlargement, erythematous, often with surface ulceration secondary to trauma. This is a report of a rare case in which a specific subtype of T-cell lymphoma appeared in the oral cavity.
Lymphomas are defined as heterogeneous malignancies of the lymphatic system characterized by a proliferation of lymphoid cells or their precursors. According to the WHO definition,1 lymphomas are classified as non-Hodgkin’s lymphoma (NHL) or Hodgkin’s lymphoma (HL). Non-Hodgkin’s lymphomas represent 86% of all lymphomas.
NHL is the second most common oropharyngeal malignancy after squamous cell carcinoma. Oral lymphomas account for 2.2% of all head and neck malignancies, 5% of salivary gland tumours, 3.5% of intraoral malignancies and 2.5% of all cases of lymphomas.2
There is an increasing incidence of NHL in patients with autoimmune disease, including coeliac disease, immune suppression from HIV, rheumatoid arthritis and Sjögren’s syndrome.3 Diagnosis of NHL in the oral cavity may result from mucosal tissue or gingival swelling, or masses.4 When major salivary glands are involved, they are commonly the first area of clinical manifestation of disease, and disease is often confined to the salivary glands.
This case report describes a patient with T-cell lymphoma affecting the right labial commissure.
A 57-year-old man presented to our hospital in 2006 for diagnosis, evaluation and treatment of a small lesion on the right labial commissure (Fig 1). He complained of a painless ulcer that had initially grown very slowly. On examination there was a small irregular ulcer with maximum diameter of 0.5 cm. The lesion had caused the patient difficulty in speaking and eating because it often bled. The lip and skin around the lesion was of normal colour and texture. On palpation, the lesion was tender. No lymphadenitis was found in the neck and cervical region. On admission, the patient’s physical condition was satisfactory. The patient’s medical history showed he was a non-drinker and a non-smoker, and there was no history of trauma or infection. The patient reported that he was receiving treatment for hypertension and hypercholesterolaemia.
The clinical differential diagnosis included angular cheilitis, traumatic lesion, infectious, vesiculo-bullous and other neoplastic diseases. An incisional biopsy was obtained under local anaesthesia through an incision with a scalpel punch (Fig 2). Histologic examination showed superficial squamous epithelium with acanthosis and hyperkeratosis. A linfoid infiltrate was pre-sent in the connective tissue. This was characterized by small elements with irregular nuclei and poor cytoplasm.
Immunohistochemical investigation showed the pleomorphic cells to exhibit a marker profile consistent with CD30 + TNHL (positive for CD30 and T-cell markers CD2, CD3 and CD4) (Figs 3–4). It was at this time that the diagnosis of T-cell NHL was made. For further staging, the patient was referred to the Department of Oncology and Haematology. Regular blood tests were normal. Lactate dehydrogenase (LDH) levels were 331 IU/L (normal range 100–250 IU/L). The patient’s ECOG (Eastern Cooperative Oncology Group) performance status5 was zero. Physical examinations as well as computed tomography scans of the thoracic and abdominal regions and sonography of the abdominal region, showed no signs of enlarged lymph nodes or hepatosplenomegaly and did not show any further involvement of the disease. Trephine biopsy of the bone marrow of the pelvis showed a normal haemopoietic pattern of the red and white blood-forming cells and no infiltration of NHL cells.
Viral markers were negative. According to Ann Arbor staging,6 the patient was at stage IE (clinical stage of extranodal localization).
As lymphoma was not found in other locations, no systemic treatment was applied. Oncologists decided to perform local radiotherapy. In order to protect the soft and hard tissues around the lesion, an oral device with buccal leaded protection was built (Fig 5).
Three months after radiotherapy, a complete remission of clinical signs was achieved. No evidence of local recurrence arose after more than three years of extensive follow-up (Fig 6). The patient was taught to self-examine for preventive care and returns every three months for follow-up.
Lymphomas are common and heterogeneous malignancies characterized by proliferation of B-cells (most commonly), T-cells, or natural killer cells, which originate in lymph nodes or extranodal lymphatic tissues.7 Non-Hodgkin’s lymphoma ranges from being relatively indolent to highly aggressive, and may be fatal. It may affect patients at any age. The incidence rate increases exponentially between 20 and 79 years, and is higher in men than women.
Primary extranodal site involvement is seen in 10% to 35% of cases at initial diagnosis and upwards of 50% during the course of the disease. These sites include the skin, kidneys, gastrointestinal tract, testes (in men), and bone.8
Malignant lymphoma of the oral cavity has been described previously although reports on the occurrence of intraoral extranodal T-cell lymphomas are scarce.9 Oral lesions may appear as a painless enlargement, erythematous, often with surface ulceration secondary to trauma. In general, the oral manifestation of NHL occurs secondary to a more widespread distribution throughout the body. In our case, the perioral manifestation of the disease was the only site.
When oral soft tissue lesions first appear, they are relatively soft and often have an overlying ulceration, and are often characterized by an absence of other symptoms. If bone is the primary site, tooth mobility and alveolar bone loss are often noted. Pain, swelling, numbness of the lip and pathologically related fractures may be associated with the bone lesion.10
The cause of NHL is still unclear. Viruses have been suggested as a potential cause of the disease. An increased rate of lymphoma in patients who receive immunosuppressive therapy and patients who are congenitally immunosuppressed has been reported.11 Prognosis and outcome have been related to the expression levels of specific molecules, e.g., higher expression of CD38 in chronic lymphocytic leukaemia is associated with decreased risk.12
Serum concentration of lactate dehydrogenase (LDH) is an important predictor of survival in NHL as a high level of this enzyme indicates high tissue damage and lymphoma relapse or renewed growth.13 Staging of NHL is important for both guiding therapy and determining prognosis. The patient in this case report was at stage IE (clinical stage of extranodal localization). After treatment, he was fully active and able to resume all pre-disease activities without restriction.
Dentists can play an important role in the early detection of the disease by understanding the signs of lymphomas. To establish an accurate diagnosis, microscopic evaluation and immunohistochemical staining are necessary. Extensive follow-up is needed to prevent a relapse.